| 2. |
- Linnankivi, T, et al.
(författare)
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Cerebroretinal microangiopathy with calcifications and cysts.
- 2006
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Ingår i: Neurology. - : Ovid Technologies (Wolters Kluwer Health). - 0028-3878 .- 1526-632X. ; 67:8, s. 1437-43
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Extensive cerebral calcifications and leukoencephalopathy have been reported in two rare disorders Coats plus and leukoencephalopathy with calcifications and cysts. In the latter, a progressive formation of parenchymal brain cysts is a special feature, whereas Coats plus is characterized by intrauterine growth retardation, bilateral retinal telangiectasias and exudations (Coats disease), sparse hair, and dysplastic nails without cyst formation. METHODS: We identified 13 patients, including two pairs of siblings, with extensive cerebral calcifications and leukoencephalopathy. We reviewed clinical, ophthalmologic, radiologic and neuropathologic data of seven deceased patients and studied five patients prospectively. RESULTS: Eleven patients were small for gestational age; the other symptoms emerged from infancy to adolescence. All patients had neurologic symptoms including seizures, spasticity, dystonia, ataxia, and cognitive decline. Progressive intracerebral calcifications involved deep gray nuclei, brainstem, cerebral and cerebellar white matter, and dentate nuclei and were accompanied by diffuse white matter signal changes and, in five patients, cerebral cysts. Eleven patients had retinal telangiectasias or angiomas. Additional features were skeletal and hematologic abnormalities, intestinal bleeding, and poor growth. Neuropathologic examination showed extensive calcinosis and abnormal small vessels with thickened, hyalinized wall and reduced lumen. CONCLUSIONS: Our data suggest that Coats plus syndrome and leukoencephalopathy with calcifications and cysts belong to the same spectrum. The primary abnormality seems to be an obliterative cerebral angiopathy involving small vessels, leading to dystrophic calcifications via slow necrosis and finally to formation of cysts and secondary white matter abnormalities.
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| 3. |
- Toivonen, M, et al.
(författare)
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Acute Acoustic Trauma after Exposure to Assault Rifle Noise among Conscripts in the Finnish Defence Forces-A Population-Based Survey
- 2023
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Ingår i: International journal of environmental research and public health. - : MDPI AG. - 1660-4601. ; 20:4
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Tidskriftsartikel (refereegranskat)abstract
- Conscripts are exposed to various sources of impulse noise despite hearing protection recommendations. The aim of this study was to investigate the frequency of acute acoustic trauma (AAT) among conscripts after exposure to assault rifle noise in the Finnish Defence Forces (FDF). This nationwide population-based cohort comprised all conscripts (>220,000) in the FDF during the years 1997–2003 and 2008–2010. We included those who claimed to have AAT symptoms from assault rifle noise during the study periods. During the investigated 10 years, 1617 conscripts (annual variation, 75–276) experienced a new hearing loss due to AAT. Altogether, 1456 (90%) of all AAT-induced hearing losses were caused by rifle-caliber weapons and 1304 (90%) of them when firing a blank cartridge. There was no clear diminishing trend in the annual numbers of AATs. In 1277 (88%) incidents, no hearing protector was used. Tinnitus was the most prominent symptom. Hearing losses after AAT were typically mild, but serious deficits also occurred. In conclusion, we found that 0.7–1.5% of the conscripts experienced an AAT during their service in the FDF. Most incidents occurred when firing a blank cartridge with a rifle-caliber weapon and with no hearing protector in use.
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